scholarly journals Safety and Tolerability of a Rapid Transition From Intravenous Treprostinil to Oral Selexipag in Three Adolescent Patients With Pulmonary Arterial Hypertension

2021 ◽  
Vol 26 (5) ◽  
pp. 512-516
Author(s):  
Elizabeth Colglazier ◽  
Angelica J. Ng ◽  
Claire Parker ◽  
Hythem Nawaytou ◽  
Jeffrey R. Fineman
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Adverse Events ◽  
Pediatric Patients ◽  
Case Series ◽  
Inpatient Setting ◽  
Inpatient Hospitalization ◽  
Adolescent Patients ◽  
Rapid Transition ◽  
Pulmonary Arterial

There is limited published experience with transitioning pediatric patients from parenteral treprostinil to oral selexipag therapy. In addition, published transitions have typically been protracted, taking several weeks to complete. We present a case series of 3 adolescent patients who were transitioned from parenteral treprostinil to oral selexipag over a 5- to 7-day period. Their clinical courses leading up to the transitions are summarized and their outcomes are described. The 3 patients were successfully rapidly transitioned during an inpatient hospitalization without any observed adverse events or prostacyclin-related side effects. We conclude that when indicated rapid transition of parenteral to oral prostacyclin therapy may be performed safely in adolescents in an inpatient setting.

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

scholarly journals Transition from parental prostacyclin to selexipag: a case series of five pulmonary arterial hypertension patients

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401986216 ◽  
Author(s):  
Nathan Holthaus ◽  
Kurt W. Prins ◽  
Lauren Rose ◽  
Sasha Prisco ◽  
Marc Pritzker ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Cardiac Index ◽  
Arterial Hypertension ◽  
Case Series ◽  
Central Line ◽  
Functional Class ◽  
Sociocultural Factors ◽  
Pulmonary Vascular Disease ◽  
Inpatient Setting ◽  
Pulmonary Arterial

Parental prostacyclin is the only therapy with a proven survival benefit in pulmonary arterial hypertension (PAH). However, some patients are unable to tolerate continuous prostacyclin infusion because of central line infection, side effects, or sociocultural factors. Selexipag is a recently approved prostacyclin receptor agonist that is able to blunt PAH disease progression. Although in the same molecular pathway, the interchangeability of selexipag with prostacyclin infusions is relatively unexplored. Here, we present a case series of five stable PAH patients who were functional class (FC) I or II that were transitioned from prostacyclin infusion to selexipag using a standardized protocol in the inpatient setting. We show that the transition to selexipag in five highly selected patients was tolerated with no significant changes in FC, minimal changes in pulmonary vascular disease severity, and no significant PAH-related complications. However, there was a trend for a reduction in cardiac index after transition to selexipag. These data suggest that a transition from prostacyclin infusion to selexipag can be achieved in clinically stable PAH patients who are unable to tolerate continuous prostacyclin infusion. However, this approach should only be selectively implemented at specialized centers with close follow-up due to the trend for a reduction in cardiac index after transition to selexipag.

scholarly journals Palliative balloon atrial septostomy in two pediatric patients with severe pulmonary arterial hypertension requiring extracorporeal membrane oxygenation support

2020 ◽  
Vol 10 (3) ◽  
pp. 004947552095973
Author(s):  
Samantha L. Brackett ◽  
Nina Deutsch ◽  
Chinwe Unegbu
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Extracorporeal Membrane Oxygenation ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Case Series ◽  
Balloon Atrial Septostomy ◽  
Membrane Oxygenation ◽  
Atrial Septostomy ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a pernicious disease with a diverse etiology in the pediatric population. Despite the increased availability of drug therapies, pulmonary arterial hypertension continues to cause significant morbidity and mortality. In pediatric patients with severe pulmonary arterial hypertension who have failed medical therapy, a few studies have demonstrated the role of balloon atrial septostomy as a bridge to lung transplantation or a means of improving symptomatology. However, no data exists on the utilization of balloon atrial septostomy as a palliative intervention to wean from extracorporeal membrane oxygenation (ECMO) when all other therapies are exhausted. Here we describe a case series of two pediatric patients with severe pulmonary arterial hypertension, requiring ECMO support, who were successfully weaned from ECMO following balloon atrial septostomy.

scholarly journals EXPRESS: The transition from Parenteral Prostacyclins to Selexipag: Safety and Feasibility in Select Patients

2021 ◽  
pp. 204589402110366
Author(s):  
Nael Aldweib ◽  
Nate Verlinden ◽  
Hayah Kassis-george ◽  
Amresh Raina
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Series ◽  
Outpatient Setting ◽  
Limited Data ◽  
Standardized Protocol ◽  
Pulmonary Arterial

There is limited data regarding the feasibility of transitioning from intravenous prostacyclins to selexipag in pulmonary arterial hypertension (PAH) patients. We present a case series of successful transitions from intravenous prostacyclins to selexipag in the majority of carefully selected five stable PAH patients using a standardized protocol in the outpatient setting.

scholarly journals Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series

2017 ◽  
Vol 7 (3) ◽  
pp. 741-746 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Case Series ◽  
Response To Treatment ◽  
Tolerability Profile ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial

Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH-CTD who have not achieved a satisfactory clinical response to a PDE-5i.

scholarly journals Occult catheter rupture causing episodic symptoms in a patient treated with epoprostenol

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774805 ◽  
Author(s):  
Barbara L. LeVarge ◽  
Anica C. Law ◽  
Blanche Murphy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of unexplained hypotension and lightheadedness in a patient receiving intravenous epoprostenol for several years, ultimately determined to be due to an unusual catheter complication, not previously described in this population.

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